Pulmonary Manifestations of Neuromuscular Diseases.

Neuromuscular disorders comprise a phenotypically diverse group of inherited and acquired diseases; however, they share common pathophysiologic mechanisms which produce significant respiratory complications. Respiratory and bulbar muscle weakness gives rise to ineffective cough, swallowing dysfunction results in aspiration-related lung disease, and abnormal muscle tone produces chest wall deformities - all of which ultimately leads to repeated chest infections and chronic respiratory failure. In this article, the authors describe the respiratory manifestations of neuromuscular disease and their underlying pathophysiological mechanisms. This review also highlights the diagnostic and management tools recommended for acute and chronic care. Aggressive pulmonary management is the most impactful at reducing the overall morbidity and improving the quality of life of children with neuromuscular disorders, at least while definitive molecular and gene replacement therapies remain elusive.

16S community profiling identifies proton pump inhibitor related differences in gastric, lung, and oropharyngeal microflora.

OBJECTIVES: To test the hypothesis that proton pump inhibitor (PPI) use results in changes in gastric microflora which, through full column reflux, results in lung and oropharyngeal microflora changes. STUDY DESIGN: We performed a prospective, cross-sectional cohort study of 116 children (57 off and 59 on PPIs) undergoing simultaneous bronchoscopy and upper endoscopy for the evaluation of chronic cough. We performed 16S sequencing on gastric, bronchoalveolar lavage, and oropharyngeal fluid. Fifty patients also underwent multichannel intraluminal impedance testing. RESULTS: Streptococcus was more abundant in the gastric fluid of patients taking PPIs, and there was a significant correlation with PPI dose (mg/kg/d) and abundance of gastric Streptococcus (P = .01). There was also a significant difference in the abundance of oropharyngeal Streptococcus in patients treated with PPI. Eight unique bacterial genera were found in the gastric and lung fluid but not in the oropharyngeal suggesting exchange between the 2 sites and 2 of the 8 (Lactococcus, Acinetobacter) were more abundant in patients with more full column reflux, suggesting direct aspiration. Principal component analysis revealed greater overlap between gastric and lung than oropharyngeal microflora. CONCLUSIONS: PPI use was associated with differences in gastric, lung, and oropharyngeal microflora. Although microflora exchange can occur between all 3 sites, gastric and lung microflora are more closely related, and the mechanism of exchange between sites may be aspiration of full column reflux.

Changes in gastric and lung microflora with acid suppression: acid suppression and bacterial growth.

IMPORTANCE: The use of acid suppression has been associated with an increased risk of upper and lower respiratory tract infections in the outpatient setting but the mechanism behind this increased risk is unknown. We hypothesize that this infection risk results from gastric bacterial overgrowth with subsequent seeding of the lungs. OBJECTIVES: To determine if acid-suppression use results in gastric bacterial overgrowth, if there are changes in lung microflora associated with the use of acid suppression, and if changes in lung microflora are related to full-column nonacid gastroesophageal reflux. DESIGN, SETTING, AND PARTICIPANTS: A 5-year prospective cohort study at a tertiary care center where children ages 1 to 18 years were undergoing bronchoscopy and endoscopy for the evaluation of chronic cough. Acid-suppression use was assessed through questionnaires with confirmation using an electronic medical record review. MAIN OUTCOMES AND MEASURES: Our primary outcome was to compare differences in concentration and prevalence of gastric and lung bacteria between patients who were and were not receiving acid-suppression therapy. We compared medians using the Wilcoxon signed rank test and determined prevalence ratios using asymptotic standard errors and 95% confidence intervals. We determined correlations between continuous variables using Pearson correlation coefficients and compared categorical variables using the Fisher exact test. RESULTS: Forty-six percent of patients taking acid-suppression medication had gastric bacterial growth compared with 18% of untreated patients (P = .003). Staphylococcus (prevalence ratio, 12.75 [95% CI, 1.72-94.36]), Streptococcus (prevalence ratio, 6.91 [95% CI, 1.64-29.02]), Veillonella (prevalence ratio, 9.56 [95% CI, 1.26-72.67]), Dermabacter (prevalence ratio, 4.78 [95% CI, 1.09-21.02]), and Rothia (prevalence ratio, 6.38 [95% CI, 1.50-27.02]) were found more commonly in the gastric fluid of treated patients. The median bacterial concentration was higher in treated patients than in untreated patients (P = .001). There was no difference in the prevalence (P > .23) of different bacterial genera or the median concentration of total bacteria (P = .85) in the lungs between treated and untreated patients. There were significant positive correlations between proximal nonacid reflux burden and lung concentrations of Bacillus (r = 0.47, P = .005), Dermabacter (r = 0.37, P = .008), Lactobacillus (r = 0.45, P = .001), Peptostreptococcus (r = 0.37, P = .008), and Capnocytophagia (r = 0.37, P = .008). CONCLUSIONS AND RELEVANCE: Acid-suppression use results in gastric bacterial overgrowth of genera including Staphylococcus and Streptococcus. Full-column nonacid reflux is associated with greater concentrations of bacteria in the lung. Additional studies are needed to determine if acid suppression-related microflora changes predict clinical infection risk; these results suggest that acid suppression use may need to be limited in patients at risk for infections.

Safety, pharmacokinetics, and preliminary assessment of efficacy of mecasermin (recombinant human IGF-1) for the treatment of Rett syndrome.

Rett syndrome (RTT) is a severe X-linked neurodevelopmental disorder mainly affecting females and is associated with mutations in MECP2, the gene encoding methyl CpG-binding protein 2. Mouse models suggest that recombinant human insulin-like growth factor 1 (IGF-1) (rhIGF1) (mecasermin) may improve many clinical features. We evaluated the safety, tolerability, and pharmacokinetic profiles of IGF-1 in 12 girls with MECP2 mutations (9 with RTT). In addition, we performed a preliminary assessment of efficacy using automated cardiorespiratory measures, EEG, a set of RTT-oriented clinical assessments, and two standardized behavioral questionnaires. This phase 1 trial included a 4-wk multiple ascending dose (MAD) (40-120 µg/kg twice daily) period and a 20-wk open-label extension (OLE) at the maximum dose. Twelve subjects completed the MAD and 10 the entire study, without evidence of hypoglycemia or serious adverse events. Mecasermin reached the CNS compartment as evidenced by the increase in cerebrospinal fluid IGF-1 levels at the end of the MAD. The drug followed nonlinear kinetics, with greater distribution in the peripheral compartment. Cardiorespiratory measures showed that apnea improved during the OLE. Some neurobehavioral parameters, specifically measures of anxiety and mood also improved during the OLE. These improvements in mood and anxiety scores were supported by reversal of right frontal alpha band asymmetry on EEG, an index of anxiety and depression. Our data indicate that IGF-1 is safe and well tolerated in girls with RTT and, as demonstrated in preclinical studies, ameliorates certain breathing and behavioral abnormalities.

The utility of endoscopy and multichannel intraluminal impedance testing in children with cough and wheezing.

BACKGROUND: Gastroesophageal reflux (GER) has been implicated as a causal factor in respiratory disease but prior studies have focused on the role of acid alone in the genesis of symptoms. Prior studies have relied on pH probe testing but this is blind to non-acid reflux which has been implicated in the genesis of extraesophageal symptoms. The objective of this prospective, cross-sectional study is to determine the utility of gastroesophageal reflux testing, including multichannel intraluminal impedance with pH (pH-MII) and upper gastrointestinal endoscopy (EGD), in the child with intractable cough and wheezing. We hypothesize that there is a high rate of pathologic reflux testing in these patients. METHODS: Children ages 1-18 with chronic cough and wheezing who were undergoing bronchoscopy for the evaluation of cough and wheezing were recruited into this prospective, cross-sectional study. They underwent identical reflux testing with pH-MII and EGD at the time of bronchoscopy. Reflux burden, symptom association, and rates of esophageal pathology were determined. Results 58% of patients had abnormal reflux testing; 67% of patients had an abnormal pH-MII test and 32% of patients had abnormal esophageal biopsies. The most common pH-MII abnormality was an abnormal symptom association between cough and reflux and the most common endoscopic abnormality was reflux esophagitis. Seven percent of patients presenting only with cough were diagnosed with eosinophilic esophagitis. CONCLUSIONS: There is a high yield to reflux testing in children with chronic cough and wheezing.

Obstructive sleep apnea in infancy: a 7-year experience at a pediatric sleep center.

PURPOSE: To investigate the common indications for polysomnogram (PSG) associated co-morbid conditions, evaluation strategies, treatment options, and outcomes in a series of infants diagnosed with obstructive sleep apnea (OSA) by a PSG. METHODS: Retrospective chart review of infants who underwent PSG over a 7-year period was done. Infants with PSG diagnosed OSA were included in this study. RESULTS: A total of 97 infants (59 males, mean age 4.6 months, standard deviation 3.3 months) were diagnosed with OSA (AHI ≥ 1/hr) based on PSG. The most common indication for PSG in infants were excessive snoring (53%) followed by nocturnal desaturations (24%). Associated co-morbid conditions included gastro-esophageal reflux (30%), laryngomalacia (24%), and craniofacial abnormalities (16%). Genetic abnormalities were found in 53%, of which trisomy 21 was the most common. Surgical treatments were employed in 36% and oxygen therapy in 15%. Thirty-eight patients were followed up with a repeat sleep study after a median interval of 8 months (range 1-24 months), of whom 26/38 had resolution of symptoms. Twenty-seven patients (28%) were followed clinically after a mean interval of 5 months of intervention (range, 1-34.5 months), in whom the symptoms resolved in 23/27 patients. Seven patients were deceased at review. Causes of death included status epilepticus, respiratory failure, hepatic failure, kidney failure, or unknown causes. CONCLUSION: The etiologies of OSA in infants are different when compared to older children. PSG is feasible and a valuable tool in the diagnosis of OSA in infants and may help determine timely and appropriate evaluation and interventions. Clinical improvement in symptoms and resolution of PSG parameters were noted following medical and/or surgical interventions. Prospective studies need to be done to ascertain the long-term outcome of infants diagnosed with OSA to assess the benefits of early intervention on their neurocognitive development.

Prevalence of pediatric periodic leg movements of sleep after initiation of PAP therapy.

INTRODUCTION: Positive-airway-pressure (PAP) is a treatment for obstructive-sleep-apnea (OSA). In adults, initiation of PAP-therapy may unmask periodic-limb movements-of-sleep (PLMS). We present a series of children in whom PLMS was aggravated or induced following initiation of PAP therapy. METHODS: Retrospective analysis was performed on children who had PAP-studies performed over a 3-year period. Children with OSA without significant PLMS on baseline studies and who then showed a periodic-limb-movement-index (PLMI) (>5/hr) during titration with PAP-therapy were identified. Charts were reviewed for demographics, OSA severity based on apnea hypopnea index (AHI), PLMI, PLM-arousal-index (PLMAI) and pressures titrated. RESULTS: Two hundred fourteen PAP-titration studies (151 on continuous positive airway pressure [CPAP] and 63 on bilevel positive airway pressure [BiPAP]) were done. Eleven (10 on CPAP, 1 on BiPAP) met study criteria. Eight patients were boys. The median age was 12 years (range 6 months-18 years). On baseline studies, median AHI was 5/hr (range 1.5-32/hr), median PLMI was 1/hr (range 0-4/hr) and median PLMAI 0.3/Hr (range 0-2/hr). On titration studies, pressures ranged from 4 to 14 cm of water, median AHI was 0.5/hr (range 0-1), median PLMI was 12/hr (range 5-55/hr) and median PLMAI 9/hr (range 0-25). PLMS were seen predominantly during N1, N2 sleep and on PAP of >7 cm of water. One patient had resolution of PLMS at 1-year follow-up. CONCLUSION: PLMS were seen in 5.1% of children after initiation of PAP. OSA may mask PLMS, which appears as breathing improves. Alternatively, PAP may unmask or induce PLMS. PLMS may potentially be a cause of treatment failure in children on recent-onset PAP therapy.

Intraesophageal pressure recording improves the detection of cough during multichannel intraluminal impedance testing in children.

BACKGROUND: One of the primary reasons for referral for reflux testing is to correlate reflux events with symptoms such as cough. Adult studies have suggested that symptom recording is flawed and pediatricians feel this is an even more significant problem because there may be errors in both parental and patient reports. We hypothesize that intraesophageal pressure recording (IEPR) provides an objective method to identify coughs during reflux testing in children. METHODS: We recruited 20 children undergoing multichannel intraluminal impedance with pH (pH-MII) testing for the evaluation of cough. We placed simultaneous intraesophageal pressure and pH-MII catheters. Tracings were blindly scored by 2 observers without knowledge of patient/parent symptom report. After the blinded scoring, patient/parent report of symptoms was recorded. RESULTS: Ninety-four percent of all coughs were detected by IEPR and only 48% of all coughs were reported by patients/parents. The mean time from the IEPR cough to the patient/parent cough was 11±16 seconds. Using IEPR as the criterion standard for the detection of cough, the sensitivity of patient report for the detection of cough is 46%. Using varying symptom windows because of the increased precision of IEPR, the number of patients with a positive symptom index could be reduced from 30% to 0%, preventing children from being falsely categorized as having reflux-related lung disease. CONCLUSIONS: Parental and patient symptom recording in children is inadequate for making the diagnosis of reflux-related lung disease. If patients undergo pH-MII testing for reflux-related cough, IEPR should become the new standard by which to correlate reflux with cough.

"Nocturnal seizures" in idiopathic pulmonary arterial hypertension.

The usual differential diagnoses of nocturnal events in children include parasomnias, nocturnal seizures, nocturnal reflux (Sandifer syndrome), hypnic jerks, periodic limb movements of sleep, and sleep disordered breathing. We report a previously healthy young girl who presented to the sleep clinic for evaluation of nocturnal events which were diagnosed as medically refractory nocturnal seizures. It was not until a syncopal event occurred in the daytime, which prompted referral for cardiac evaluation, the diagnosis of idiopathic pulmonary arterial hyper-tension (IPAH) was made. Sleep physicians should consider IPAH in the differential diagnosis of nocturnal events in children.

The diagnostic yield of pediatric polysomnography based on the professional background of referring physicians.

INTRODUCTION: Polysomnography (PSG) is the gold standard in diagnosis of sleep-disordered breathing but is labor intensive and expensive. We assessed PSG outcomes based on physician specialty and whether prior referral to sleep clinics reduces costs for children with sleep problems. METHODS: Retrospective analysis of PSGs was performed by demographics, physician specialization, PSG indication, and results. Average costs of evaluating PSGs ordered by primary care physicians (PCPs) versus initial referral to sleep specialists were compared. RESULTS: A total of 493 PSGs were performed between June 2010 and December 2010. Most referrals came from sleep physicians (31%), otolaryngologists (24%), and PCPs (18%). Forty-four percent PSGs ordered by PCPs were normal versus 22% by sleep physicians (odds ratio = 2.8, P < .05). Average cost of PSGs ordered by PCPs was $3053.54/patient versus $917.85/patient for sleep specialist referral. CONCLUSION: PSGs ordered by PCPs were more likely to be normal than PSGs by sleep physicians. Sleep clinic assessment before PSGs may assist in appropriate service utilization, improve delivery of care, and reduce health care costs by using these tests appropriately.

The maintenance of wakefulness test in pediatric narcolepsy.

Objective tools are needed to assess the response to treatment in pediatric narcolepsy. This article presents a single-center experience documenting the use of the maintenance of wakefulness test (MWT) in a pediatric series. This study reviewed the charts of children with narcolepsy who had an MWT performed between January 2008 and June 2012. A cutoff was used for mean sleep latency: <8 minutes for inadequate control of hypersomnia, and >20 minutes to indicate adequate control on medications. Thirteen tests were performed on 10 children (median age 15.8 years, range 8.7-20.3 years) with narcolepsy, of which six had cataplexy and three were boys. Comorbid conditions included Prader-Willi syndrome, bipolar affective disorder, and epilepsy (n = 1 each). The median mean sleep latency for all studies was 16 minutes (range 5.8-40 minutes). Sleep-onset rapid eye movement sleep events were seen in three of 13 studies. In seven patients, findings from the MWT resulted in changes in management. These data suggest that the MWT may be a useful and feasible test for assessing response to treatment in children with narcolepsy. Future research is needed to obtain normative MWT data on children with and without narcolepsy.

MRI findings and sleep apnea in children with Chiari I malformation.

Chiari I malformation is characterized by downward herniation of the cerebellar tonsils through the foramen magnum. Scant data are available on the clinical course, relationship to the extent of herniation on magnetic resonance imaging in Chiari I malformation and the presence of sleep-disordered breathing on polysomnography. Retrospective analysis was performed looking at polysomnographic findings of children diagnosed with Chiari I malformation. Details on how Chiari I malformation was diagnosed, brainstem magnetic resonance imaging findings, and indications for obtaining the polysomnogram in these patients were reviewed. We also reviewed available data on children who had decompression surgery followed by postoperative polysomnography findings. Twenty-two children were identified in our study (11 males, median age 10 years, range 1 to 18). Three had central sleep apnea, five had obstructive sleep apnea, and one had both obstructive and central sleep apnea. Children with sleep-disordered breathing had excessive crowding of the brainstem structures at the foramen magnum and were more likely to have a greater length of herniation compared with those children without sleep-disordered breathing (P = 0.046). Patients with central sleep apneas received surgical decompression, and their conditions were significantly improved on follow-up polysomnography. These data suggest that imaging parameters may correlate with the presence of sleep-disordered breathing in children with Chiari I malformation.

Higher rate of bronchoalveolar lavage culture positivity in children with nonacid reflux and respiratory disorders.

The aim of the study was to determine if children with chronic cough or wheezing and with more full-column, nonacid reflux have a higher likelihood of a positive bronchoalveolar lavage fluid culture. In a prospective study of 46 children with cough who were undergoing endoscopy, multichannel intraluminal impedance testing, and bronchoscopy, we found that patients who had a positive culture had significantly more full-column, nonacid gastroesophageal reflux than those who had a negative culture.

Green tea epigallo-catechin-galleate ameliorates the development of obliterative airway disease.

Lung transplantation has the worst outcome compared to all solid organ transplants due to chronic rejection known as obliterative bronchiolitis (OB). Pathogenesis of OB is a complex interplay of alloimmune-dependent and -independent factors, which leads to the development of inflammation, fibrosis, and airway obliteration that have been resistant to therapy. The alloimmune-independent inflammatory pathway has been the recent focus in the pathogenesis of rejection, suggesting that targeting this may offer therapeutic benefits. As a potent anti-inflammatory agent, epigallo-catechin-galleate (EGCG), a green tea catechin, has been very effective in ameliorating inflammation in a variety of diseases, providing the rationale for its use in this study in a murine heterotopic tracheal allograft model of OB. Mice treated with EGCG had reduced inflammation, with significantly less neutrophil and macrophage infiltration and significantly reduced fibrosis. On further investigation into the mechanisms, inflammatory cytokines keratinocyte (KC), interleukin-17 (IL-17), and tumor necrosis factor-α (TNF-α), involved in neutrophil recruitment, were reduced in the EGCG-treated mice. In addition, monocyte chemokine monocyte chemoattractant protein-1 (MCP-1) was significantly reduced by EGCG treatment. Antifibrotic cytokine interferon-γ-inducible protein-10 (IP-10) was increased and profibrotic cytokine transforming growth factor-ß (TGF-ß) was reduced, further characterizing the antifibrotic effects of EGCG. These findings suggest that EGCG has great potential in ameliorating the development of obliterative airway disease.

Utilization of lipid-laden macrophage index in evaluation of aerodigestive disorders.

OBJECTIVES/HYPOTHESIS: The utility of the lipid-laden macrophage index (LLMI) in the evaluation of airway inflammatory processes remains controversial. There is a paucity of normative data in both pediatric and adult populations, and there is wide variability in the reported cases. The goal of this project was to review the LLMI levels in a large series of patients with a wide range of well-documented pulmonary and airway diseases (cystic fibrosis, aspiration, tracheo/bronchomalacia, recurrent pneumonia, asthma, immunosuppressed conditions, and laryngeal clefts) to develop a better understanding of the clinical utility of the LLMI. STUDY DESIGN: This study is a retrospective analysis of patients who underwent initial bronchoscopy with broncheoalveolar lavage (BAL) and had specimens sent for determination of LLMI. This study was performed at a major pediatric tertiary care medical center from April 12, 2006 to July 13, 2009. METHODS: Medical charts were reviewed for major diagnoses, indications for bronchoscopy, the side of lung (right versus left), documented aspiration, suspected aspiration, neutrophil counts, and patient age. These qualitative and quantitative variables were considered in relation to the LLMI. RESULTS: The cohort of patients examined were 4.9 ± 4.3 years (4 days to 18 years of age). Forty-two percent were female and 58% were male. The highest median LLMI was in immunocompromised patients with a median of 78. Recurrent pneumonia, aspiration, tracheo/bronchomalacia, and cystic fibrosis had median LLMI levels of 55, 49, 40, and 50, respectively. There was a wide range of LLMI within each primary diagnosis. There was no significant correlation between neutrophils percentage in the BAL and LLMI. There were no significant differences between BAL specimens obtained from the right or left side. CONCLUSIONS: To our knowledge, this is the largest series of patients reported in the literature to have an LLMI compared with underlying diagnoses. Based on our data and analyses, there is wide variability between the range of LLMI and the primary diagnosis. As such, the diagnostic utility of the LLMI is limited and should be interpreted with caution.

Streptococcus milleri group pleuropulmonary infection in children: computed tomographic findings and clinical features.

OBJECTIVE: Streptococcus milleri group streptococci have recently been increasingly recognized as important pulmonary pathogens, but their imaging features have not been well documented in children. We have recently observed a number of cases of this infection among pediatric patients at our tertiary care, children's hospital. Our purpose was to investigate the computed tomographic (CT) findings and clinical features of S. milleri group pleuropulmonary infection in children. MATERIALS AND METHODS: We used our hospital information system to identify all consecutive pediatric patients (<18 years of age) who had both a microbiologically proven S. milleri group infection and a chest CT scan between December 1996 and May 2009. Each scan was systemically reviewed by 2 pediatric radiologists for pleural and lung parenchymal abnormalities. Pleural effusions were classified as either simple or complex and correlated with results of pleural fluid analysis. Computed tomographic findings were compared with chest radiographic findings in the subset of patients who underwent radiography within 24 hours of CT. Microbiological data, risk factors, immune status, patient management, and clinical outcome were systematically reviewed. RESULTS: The final study cohort consisted of 15 children (6 boys and 9 girls), ranging in age from 4.2 years to 17.7 years (mean, 10.8 years). All patients were immunocompetent without recognized risk factors for this infection. Thirteen pleural effusions were identified in 10 (67%) of the 15 patients, including 10 complex and 3 simple pleural effusions. All complex effusions at CT were consistent with empyemas by pleural fluid analysis. Lung parenchymal abnormalities were identified in 7 (47%) of the 15 patients, including lung abscess in 4 patients, consolidation in 2, and multiple bilateral pulmonary nodules and lung abscesses in 1. In the subset of 7 patients with comparison radiographs, radiographic and CT findings were concordant for the detection of lung abnormalities, except one case in which consolidation was diagnosed on chest radiography, whereas CT scan showed a lung abscess. Radiographs detected all 4 complex pleural effusions seen on CT scan, although it was not possible to characterize the effusions as simple or complex on the radiographs. Interventional procedures were required in all 15 patients, most commonly thoracentesis (n = 11) and chest tube drainage (n = 9). CONCLUSIONS: In children with S. milleri group pleuropulmonary infection, CT often demonstrates complex pleural effusions and lung abscesses, which usually require interventional procedures for effective treatment.

Restless legs syndrome and periodic limb movements disorder in the pediatric population.

PURPOSE OF REVIEW: Restless legs syndrome (RLS) and periodic limb movements of sleep (PLMS) are common neurological disorders in childhood which are usually underdiagnosed. As many pediatricians and pediatric pulmonologists with interest in sleep medicine will be encountering children with RLS and PLMS in their practice, we feel a comprehensive review of these disorders from a pediatric perspective would be very important in understanding basic pathophysiology, clinical features in early diagnosis, and effective management. RECENT FINDINGS: There has recently been increased awareness about these disorders in children, and the American Academy of Sleep Medicine has recently published pediatric specific diagnostic criteria. There have also been exciting discoveries into the basic pathophysiology of these conditions which have helped to better understand these disorders. Genetics plays a very important role in their development, and current management strategies have been very successful in treatment of these conditions. SUMMARY: RLS occurs in 1 out of 120 school-age children. In many children, diagnosis is not even suspected as these children present with atypical symptoms and associated comorbid conditions. In this review, we will discuss RLS and PLMS in the pediatric population and briefly review their pathophysiology, clinical presentation, risk factors, neurobehavioral consequences, and newer diagnostic criteria along with recent advances in their management.

Multidetector computed tomography evaluation of secondary hepatopulmonary fusion in a neonate.

Hepatopulmonary fusion is a rare condition in which a dense adhesion occurs between the right lung and herniated hepatic parenchyma in patients with right-sided congenital diaphragmatic hernia. Plain radiographic and magnetic resonance imaging (MRI) findings of hepatopulmonary fusion in a neonate have been reported in a retrospective study with a small patient population and a case report. However, to our knowledge, there is no report regarding the secondary hepatopulmonary fusion (after right-sided congenital diaphragmatic hernia repair) evaluated with multidetector computed tomography (MDCT) in a neonate. We report a case of secondary hepatopulmonary fusion in a neonate, in which multiplanar and three-dimensional (3D) images were helpful in delineating the precise anatomy for preoperative evaluation. Understanding the diagnostic limitations of plain radiographs and MRI, the use of MDCT with its multiplanar and 3D imaging may emerge as a useful noninvasive imaging modality in the evaluation of possible hepatopulmonary fusion in pediatric patients with right-sided congenital diaphragmatic hernia.